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Volume 15
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Review

Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview

by
Gerardo Cazzato
1,*,
Marialessandra Capuzzolo
1,
Emilio Bellitti
2,
Giovanni De Biasi
1,
Anna Colagrande
1,
Katia Mangialardi
3,
Francesco Gaudio
3 and
Giuseppe Ingravallo
1
1
Section of Molecular Pathology, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari “Aldo Moro”, 70124 Bari, Italy
2
Anatomic Pathology Unit, “A. Perrino” Hospital, 72100 Brindisi, Italy
3
Hematology Section, Department of Emergency and Transplantation, University of Bari Medical School, 70124 Bari, Italy
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2023, 15(4), 696-706; https://doi.org/10.3390/hematolrep15040070
Submission received: 18 September 2023 / Revised: 9 November 2023 / Accepted: 4 December 2023 / Published: 8 December 2023
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Abstract

Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review and further studies that deeply investigate this entity not only in a histopathological but also molecular field. In August–September 2023, we searched MEDLINE, PubMed and Scopus for randomized controlled trials (RCTs), narrative and systematic reviews, meta-analyses, observational studies (either longitudinal or retrospective), and case series published in English in the last 25 years using the keywords BPDCN, PDCs, Blastic NK-cell lymphoma, agranular CD4+ NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm/tumor. Despite the progress made in recent years in the diagnosis and biological understanding of the disease, until 2018 there was no clear consensus regarding its treatment and the main therapeutic schemes used were based on chemotherapy regimens already used in the treatment of lymphomas, acute lymphoblastic leukemia (ALL) and/or acute myeloid leukemia (AML). In this narrative review, we address the definition and epidemiological features of BPDCN, provide the different theories on the etiopathogenesis with particular attention to the presumed cell of origin, discuss the main clinical manifestations that provide a sign of its presence, summarize the main histopathological and immunophenotypic characteristics with special attention to the most important markers, and finally, we provide some of the most effective information on the therapeutic treatment modalities of BPDCN.
Keywords: BPDCN; aggressive neoplasm; hematology; PDCs; WHO; myeloid; leukemia; lymphoma BPDCN; aggressive neoplasm; hematology; PDCs; WHO; myeloid; leukemia; lymphoma

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MDPI and ACS Style

Cazzato, G.; Capuzzolo, M.; Bellitti, E.; De Biasi, G.; Colagrande, A.; Mangialardi, K.; Gaudio, F.; Ingravallo, G. Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview. Hematol. Rep. 2023, 15, 696-706. https://doi.org/10.3390/hematolrep15040070

AMA Style

Cazzato G, Capuzzolo M, Bellitti E, De Biasi G, Colagrande A, Mangialardi K, Gaudio F, Ingravallo G. Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview. Hematology Reports. 2023; 15(4):696-706. https://doi.org/10.3390/hematolrep15040070

Chicago/Turabian Style

Cazzato, Gerardo, Marialessandra Capuzzolo, Emilio Bellitti, Giovanni De Biasi, Anna Colagrande, Katia Mangialardi, Francesco Gaudio, and Giuseppe Ingravallo. 2023. "Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview" Hematology Reports 15, no. 4: 696-706. https://doi.org/10.3390/hematolrep15040070

APA Style

Cazzato, G., Capuzzolo, M., Bellitti, E., De Biasi, G., Colagrande, A., Mangialardi, K., Gaudio, F., & Ingravallo, G. (2023). Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview. Hematology Reports, 15(4), 696-706. https://doi.org/10.3390/hematolrep15040070

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